Pediatric sarcoidosis presenting with hypertensive encephalopathy.

A 3-year-old girl with simultaneous presentation of hypertensive encephalopathy, subcutaneous nodules, hepatosplenomegaly and proteinuria was presented. The sarcoidosis was confirmed by histologic demonstration of non-caseating granulomas in the liver, left kidney, a cervical lymph node and subcutaneous nodules. The proteinuria resolved spontaneously. During the six-month period of steroid therapy, the renal mass and hepatosplenomegaly were dissolved but the cervical lymphadenopathy still persisted. The hypertensive state was difficult to control which required a multiple antihypertensive drug regimen.

Henoch-Schönlein purpura in Thailand: review of 41 children.

The clinical features of 41 Thai children with Henoch-Schönlein purpura were presented, with particular emphasis on the cutaneous features and date of onset. There were 20 boys and 21 girls, ages ranged from 2 to 15 years. All patients had palpable purpura on the lower limbs. Palpable purpura were also present on forearms and pinna in 25 (61%) and 5 (12%) respectively. Scalp edema was found in five patients. Hemorrhagic vesicles and bullae were found in only one patient. Twenty six patients (63%) had skin lesions as the first sign. Thirty four patients (83%) had skin lesions in the first four days of the clinical course. Other dominant features included were abdominal pain, arthralgia and nephritis 73, 66 and 39 per cent respectively. The pertinent literature is reviewed.

Collodion baby: the out-come of long-term follow-up.

About 10 Thai collodion babies were treated at the Department of Pediatrics, Faculty of Medicine, Chulalongkorn University from 1970 to 1990. Most of them were premature and small for gestational age infants. The sex incidence of males to female was 3:2. Only half of the cases had a family history of ichthyosis. The severity of thickness and tightness of the collodion membranes varied from mild to severe. The clinical and histopathologic findings revealed 5 cases of lamellar ichthyosis (50%), of which one case had episodes of pustular psoriasis. One case evolved to be ichthyosis vulgaris 10 per cent, one case had palmoplantar hyperkeratosis (10%), one case had been apparently normal up to 18 years old, one died of respiratory infection and another case was lost from follow-up. All cases of lamellar ichthyosis were associated with mental retardation with some degree of slow physical development. One severe collodion baby suffered from congenital absence of distal phalanges of toes with increased thickness of the finger nails.

Corticosteroid treatment of extensive hemangiomas: analysis of 22 cases in children.

During the past 16 years, 22 Thai infants with extensive hemangiomas which impaired bodily functions and were life-threatening, especially in Kasabach-Merrit Syndrome have been treated. The incidence of female-to-male ratio was 8:3, all of the hemangiomas appeared between birth and 3 months of age. Among the 22 infants, 11 (50%) had hemangiomas on the head and the neck, and 2 had laryngeal hemangiomas. Two cases of Kasabach-Merrit Syndrome, located at the shoulder and the thigh, were complicated with severe bleeding, infection and congestive heart failure. Ocular impairment, malocclusion, and cutaneous distortion were the most important functional problems. Three cases were associated with other congenital anomalies: intracranial angioma, Dandy Walker Syndrome and Klippel-Trenauncy Syndrome. Prednisone (2-4 mg/kg/d) was used in all cases with other symptomatic treatment. Antiplatelet aggregating and antithrombotic drugs were useful in Kasabach-Merrit Syndrome. Definite response was noted in 72.72 per cent of the patients, doubtful response in 23.32 per cent, and no response in 4.54 per cent which was better than in other reports. There were no serious side-effects of the corticosteroid treatments reported in our careful students.

Concomitant disseminated varicella and generalized staphylococcal scalded skin syndrome in a leukemic patient.

We report a case of nosocomial fatal varicella infection in a 13-year-old boy with acute lymphoblastic leukemia complicated with staphylococcal scalded skin syndrome. His underlying leukemia, immunosuppressive drugs, disseminated varicella, S. aureus colonization, and acute renal insufficiency were all contributing factors that were pathogenetically linked in the development of his generalized SSSS.